ABSTRACT
Plate 1 Lesions of the small pulmonary arterioles found in all forms of PAH, including FPPH.65 Lesion one demonstrates eccentric intimal fibrosis and medial hypertrophy (Verhoff van Gieson, magnification 100). This lesion was originally thought to represent pulmonary microembolism, but is widespread in FPPH, denoting a differing mechanism. Lesion 2 shows concentric intimal fibrosis, an onion skin-like layering of fibrotic reaction (Movat stain, magnification 100). Lesion 3 is a plexiform lesion, showing a neovascular response (hematoxylin and eosin, magnification 100). It is unclear whether these lesions are a response to upstream obstruction or are a primary lesion in PAH. (See p. 368.)
Plate 2 Lung histopathology of an infant with SP-B deficiency. The interstitium is thickened and alveolar type II cells are prominent. There are macrophages and desquamated epithelial cells mixed with eosinophilic granular material within the alveolar space. (See p. 463.)
Plate 3 Lung section from an infant with ABCA3 deficiency. The alveolar spaces are filled with macrophages, desquamated epithelial cells, and proteinaceous material. There is alveolar type II cell hypertrophy and widening of the interstitium. (See p. 466.)
Plate 4 Lung histopathology of an individual with idiopathic PAP. Note the fine granular eosinophilic material that completely fills the alveoli space and the normal appearance of the alveolar interstititum. (See p. 468.)
Plate 5 Comparison of the phenotype of tuberin-deficient and wildtype Drosophila eyes. Random gene inactivation was followed by characterization of eye phenotypes. Enlarged eyes cells in tuberin-deficient flies (b) compared to wildtype flies (a) were discovered. Phase microscopy revealed differences between normal pigmented wildtype eye cells (c, upper left), compared to enlarged, nonpigmented cells in tuberin-deficient flies (c, lower right). Bristles (d) arising from cells at the anterior edge of the wing were larger and less pigmented in those containing tuberin mutations (gig). Photomicrographs of cells in the wing revealed enlargement in those containing the tuberin (gig) mutant compared to wildtype (e) (From Ref. 48). (See p. 481.)
Plate 6 Pathology of LAM. (a) Normal lung histology; (b) Alveolar septum expanded by spindles shaped smooth muscle; (c) HMB-045 staining of smooth muscle in LAM; (d) Smooth muscle infiltration, cyst formation; tissue destruction in LAM. (See p. 485.)
